特发性肺动脉高压（IPAH）是指原因不明的肺血管阻力增加，引起持续性肺动脉高压力升高，导致评价肺动脉压力在静息状态下≥25mmHg，排除所有引起肺动脉高压的继发性因素。原发性肺动脉高压原指病因未明的肺动脉高压，目前已发现骨形成蛋白II受体基因突变等原发性肺动脉高压的病因。将以往的原发性肺动脉高压患者中，具有肺动脉高压家族史的患者归入家族性肺动脉高压，其余的即为特发性肺动脉高压。本病多见于中青年，男女发病率之比为1:2～3。
目的：特发性肺动脉高压(idiopathic pulmonary arterial hypertension,IPAH)是一种原因不明的以肺血管阻力增加引起肺动脉压力持续升高为特征的肺血管疾病,其发病机理不清,临床缺乏特异性症状和体征,致...
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...界卫生组织将原发性肺动脉高压(primary pulmonary hypertension,PPH)改称为特发性肺动脉高压(idiopathic pulmonary hypertension,IPH),是一种不明原因的肺动脉高压。
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治疗特发性肺动脉高压 idiopathic pulmonary hypertension ; IPAH
目前特发性肺动脉高压 idiopathic pulmonary artery hypertension ; IPAH
称为特发性肺动脉高压 idiopathic pulmonary arterial hypertension ; IPAH
采用特发性肺动脉高压 idiopathic pulmonary arterial hypertension ; IPAH
为特发性肺动脉高压 idiopthic pulmonry hypertension
又是特发性肺动脉高压 IPAH ; Iidiopathic Pulmonary Artery Hypertension
及特发性肺动脉高压 Idiopathic pulmonary arterial hypertension
用特发性肺动脉高压 Idiopathic Pulmonary Arterial Hypertension
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ES病人和特发性肺动脉高压病人的临床过程有显著的差异。
There are significant differences in the clinical course between patients with es and those with idiopathic pulmonary hypertension.
ES病人与那些未经治疗的特发性肺动脉高压病人比较，生存率更佳。
Patients with ES show significantly more favorable survival rates, as compared with those with untreated idiopathic pulmonary hypertension.
抗凝治疗对特发性肺动脉高压病人十分重要，但是在治疗ES方面存在争论，因为出血风险高。
Anticoagulants, which are an important part of therapy for many patients with idiopathic pulmonary hypertension, are controversial in ES, because of the high risk for bleeding complications.
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